A Shot in the Dark

 The long researched, longed for, and long planned for growth hormone medicine arrived. Before it arrived, the phone calls arrived. The doctors personal assistant, the specialty pharmacy who will deliver the meds, the nurse calling to schedule the training session. Then I called the pharmacy nurse to talk to her, and then the case manager for the GH called, and then I called the personal assistant again, and then the nurse called again to say she's on her way. Two days before the nurse came I promised the specialty pharmacy I'd be home to receive my on ice delivery. All those months of investigation on the computer and trips to doctors, and suddenly it's here and I'm not sure how this works.

The above picture of Mimi in the GH company duffle bag gives an indication of the excitement we felt when the boxes of needles, alcohol wipes, and injection pens arrived. This has been such a long awaited thing, we were really really ready.

Mimi was scared when it was time to do the first shot with the nurse. My Mom came to learn, Matt came home early from work, Max stayed quiet and watched, and the nurse instructed me as Mimi screamed. Then she laughed when it was done.

The next night I tried giving her the first on my own shot, while she slept. Lots of moms had said they do it that way, and it worked great. Mimi didn't flinch or move a muscle. I was so relieved. I found the whole thing very easy. I just had to remember to take the growth hormone out of the fridge for 20 minutes first and I had to stay up to wait til she'd been asleep a little while.

So I proceeded to do the shots, Monday night, Tuesday, Wednesday, and Thursday. Her dose allows us to be off the seventh night (6 nights shots, 1 night off). Friday night found us in Connecticut visiting my sister. Mimi had developed little dark circles under her eyes, but all else seemed fine.
By 11pm Mimi was congested and crying. She kept crying and crying and crying. Then she got a little fever. She woke up the next morning and I gave her Tylenol. She was ok, playing with her cousins and singing in the yard with all the kids.

On the drive home that afternoon, she fell asleep and then woke up at a truck stop blazing hot. I took off her undershirt and put her back in just a t-shirt and took her socks and shoes off to cool her down as we kept driving. I felt unprepared as I did not have her Endocrine docs phone number or children's Tylenol. errrrrr. She cooled down again, and by dinner was singing to everyone in Arby's. LOL

Back home she seemed ok - I thought she probably just has a virus or something. But on Tuesday afternoon, her eyelids looked weird - puffy- not too bad, but droopy. So I went through all the warnings for the drug and thought I better let her Endo know as that was listed as a side effect. I thought I was being overly concerned. She wrote me right back and said no dose tonight, and slowly start her back up on lower amounts until we reach half the starting dose.

The next day, Wednesday, I took her into her primary doctor to check her lungs as she'd had off and on fevers and coughing. No lung trouble, but wait, she has a double ear infection.

Back home and I look up the drug warnings again and there can be ear infections with somatropin (GH). Mimi had her last ear infection 14 months before, so that was a coincidence? that a week into GH she has a double ear infection?

Where last week there was ease and hopefulness, now the train of excitement has stalled and I'm holding my breath and checking her face to see if she's looking puffy on each dose level.

I don't understand if these things happening means that she's in danger or if she'll need to be on lower dosing all the time. Will she not grow as fast on it if she is lower dosed?  Will she be ok on it? I also am thinking Why is her face swelling and why are her ears infected? Why does that happen? Can someone tell me? The primary doctor said that her body is reacting to the change. I don't know...
I don't understand. I hope this can be one of those stories of "at first it was a little bit hard for her, but now she's doing soo great."


We have a busy weekend coming up, and I am trying to get things in order around the house. It felt  like this week I got behind in all my tasks and the house on any given day looked crazy. And then I remembered that it's hard to do it all, when a sick little one only wants you. And I remembered that she wanted me to hold her and read to her, and play Barbies, and library, and Frozen. And I knew she felt strange and her ears hurt her.

That's just fine, she has me. And I'm not taking my eyes off hers- with each day and each increase. .1,2...

Mouse Loves Rice

Max became involved in the Music for Everyone program this year at his school in Lancaster.
They were awarded a grant that allowed 2nd and 3rd graders to have choir classes after school this spring.

Last night they were invited to record a song for the soundtrack for a documentary film. You should have heard them all singing...it was so sweet. It was the "Mouse Loves Rice" song.

Here are some photos of the kids at the Winter Visual and Performing Arts Center and the crew...Wow...!

I'm sure he'll always remember this....!

 

 

...Meanwhile, Mimi and I waited outside in the hallway and could hear their lovely voices.

She worked on her stickers for a while and then said that she is a star and started belting out her own "Frozen" inspired song and dancing with her new moves.

She made me a little nervous there as she's singing right outside the studio door. LOL

It will be cool to see the film when it comes out.

It's a New Day

We switched Endos last month and took Mimi to Dupont. I ordered for all her records and insane history of test results to be faxed over, including all growth charts and everything else we could think of before we got there. We drove for an hour through picturesque Amish farms, negotiating the roads that were ravaged by potholes from our insanely cold winter.

Mimi had a thorough consult with a wonderful Endo who was so careful and meticulous, but also very funny and sweet to her. We walked out with a script for Growth Hormone. We also got a referral to cardiology (not messing around), one for labs for 2 weeks into GH (including a Karyotype lab thrown in...not messing again X2).  It was sort of like Disney World. Everything went smooth, was a successful appointment, and left me feeling shocked with relief.

Mimi works with Play Doh and sings with Paul Simon...

Join me for a day, and if we leave the house Mimi shall bring her player. No need to turn on the radio. She'll play "Me and Julio..." and sing along, pressing repeat until we get to our destination.

In this video she listens carefully and then the singing starts up and she joins right in- working happily and singing. My sweetie...

Pituitary Dwarfism in History

I came across these photos in looking up historical images of Growth Hormone Deficiency. I was trying to see what Mimi might look like if she did not have Growth Hormone treatments, or if they didn't work for her.
Although the term midget is quite frowned upon now, it was the term that was used in the past when someone was a proportionate dwarf. So the term "midget" came up under many of these photos. (it is correct to now refer to a short statured person as a Little Person.)

I thought it was important to show these images, as you can see the similarities in facial features between these people and children now with GHD. Because most children with GHD are treated with growth hormone today, it's hard for me to visualize what an adult with untreated congenital GHD "looks" like.

We aren't sure what the diagnosis of each individual here was. Some may have had thyroid disfunction that led to a stunting of growth. Maybe some had a tumor on the pituitary. Some may have had Laron syndrome or IGFD, some may have a malformed pituitary gland.

Pituitary dwarfs also worked as the munchkins in the Wizard of Oz.

I wonder about the lives of the people pictured, their feelings, and the thoughts of their families as well. How they viewed themselves, what it was like to walk down the street that size, back then, and even how they found their clothing. It is evident to me that many have the cherubic face that Mimi has.

 I love this card, because it shows weights and heights. Something that parents of children with growth disorders always talk about. It would be fascinating to know the actual diagnosis of the person listed, and their caloric intakes in early childhood. One parent on the facebook group I'm on mentioned her daughters doctor said she'd be in the three foot range without treatment. A Laron dwarf today averages four feet tall. These people listed on the card are very small.

Now, before beginning GH treatments, doctors might see if we as parents can try to add height to our children by increasing calories for a few months. That worked for Mimi over the past year. We're not talking huge increases in height. We're talking, "she was barely growing  at all and then we increased her calories by about 500 a day. (putting her at 1700 calories during age 2 to 3). She grew an inch suddenly." Now we're pulling teeth in the growth department again, despite what she eats. Mimi makes barely any growth hormone (averaging 3.4 on two growth hormone stimulation tests.) Now she is gaining weight, but I'm charting the growth, and the little pencil line hasn't moved much.

I wonder if these children could have been a "wee" bit taller with more food, more protein? (Mimi craves a strangely large amount of protein.) I'm talking about maybe being a few inches taller as a result of a ton more food. It  is very intense feeding Mimi 1700 calories a day, and exhausting. Did mothers cook at midnight for these children? Or would that seem odd to them? Did some mothers try it, only to feel that they might be going crazy? Stirring the pot of food on the stove by oil lamp, while their little friend waited for food- everyone else asleep in the house?! As I, in 2012-14 can barely find any information about this at all, those moms certainly didn't read that feeding more could help. It would have had to have been by instinct alone.

Dot Ivenzel here weighs 12 pounds more than Mimi and is three inches taller than Mimi now.

With no chance of a medication to help your child, and no medical information to read...did a mother go mad? I have very strange feelings about Mimi's lack of growth. Initially I felt that all eyes stared at me and wondered if I fed her. (which quite often is what people thought- and I know that because they'd ask me if I fed her! (and glare at me.) Now I go around in a kind of proud haze, sort of self made blinders to the world. Replacing my worries for what other people think with a spacey amazement for her (with gulps of concern for her health and future popping in at times.) Every season I feel like I'm fighting a magnetic pull as I put her clothes away in a bin for next year, instead of giving them to someone else- because her clothes will still fit. It doesn't feel right, but I have to self talk myself and say it's ok. Save them.

Perhaps all the mothers of the people in these photos were stoic creatures who just went with the flow, completely unworried for their Daisy, or Edward, or tiny Sophia. But surely they weren't diagnosed for years (we weren't with the advancements of today). Surely there were comments and teasing, and staring. But surely also, they had brothers and sisters who loved and protected them to pieces.

Even though it's been a long time of wondering what's going on with Mimi, we have it much easier than in the past. I can't stop looking at these adults and thinking of their moms.

Olga Nardone is pictured below, at age 18 during the Wizard of Oz.  She was 40 inches tall and one of the smallest munchkins. She died at 89.

You don't see photos like this online very often. I'm thankful to see them, and I see my daughter and other kids we've met too in these images. xo

With Love, From Our Family to Yours...

Max and Mimi at Conestoga House Gardens...remembering Summer

Just Like a Rock Star...Thank You Fresh Beat Band!

We were so excited to head to Hershey last night for something so fun and special.

We attended the Fresh Beat Band concert at the Giant Center...and I have to say, it was a beautiful sight to see that "Little Light of Mine" happier than she's ever been I think.

And for her big brother to do something so exciting. We needed a fantastic night out!

This was the official first concert experience for both kids!

They could hardly wait for the show to start!

We were really treated so well as VIP guests! Check out the fab seats and cool Fresh Beat Band wrist bands (which 24 hours later they are still wearing, after having gone to school in them, etc. !)

 Mimi was so engaged, such a big girl. She was SO excited, her eyes lit up in anticipation.

And his too...!

At times I held Mimi on my lap, and would wonder what she was thinking. Then I'd glance around at her expression, and every time she was beaming. Or giggling. At Twist. LOL

 My favorite picture of night.

Mimi made a new friend named Carly, and it was all I could do to keep her off the floor (sorry ushers! the kids have to dance!) The band sang all the favorites and were hilarious.  Needless to say, Mimi is playing "dance show" today. And Max is considering taking dance classes like hip hop!

 

 The show ended with machines shooting confetti from the stage into the air, and cool huge beach balls bounced through the audience.

After the show, we headed back to the VIP party. There were healthy snacks including pretzels and organic fruit chewies. Lots of costumes and things to play on. A dance contest, and the much anticipated photos with the band.

 Check out Max with the gang. Marina had just told him she loved his glasses. So sweet!

If you can check out a Fresh Beat show in your local city, I'd recommend it. The kids will have so much fun. What a professional group of performers. They don't miss a beat!

Thank you Fresh Beat Band! You rock!

Officially Growth Hormone Deficient

Mimi had a busy end of November, with two trips to Hershey Endocrine. On November 18, she underwent a morning long Growth Hormone Pituitary Stimulation Test- which is three and a half hours of blood draws. Awake and alert...hard enough for adult. Imagine getting this done at three years old.
If your child is having growth issues, it can take a long time to get to this test...and so for us, it was a major milestone, that we knew would lead to her diagnosis.

The results came back a week later, and we came in to hear them.

My little sweet petunia failed the test quite impressively, proving to ALL that she is Growth Hormone Deficient, and has Pituitary Dwarfism.

The two tests they ran came back in the 2 and 4 range with an average of 3.
A score of 10 or under can prove you need the $2,000-$3,000/month growth hormone shots. A low level like Mimi's shows that she really isn't creating much growth hormone at all, and the lack of GH she makes can have a significant impact on her entire body.

Is this just about being small? No, sadly no. And I love munchkins, gnomes...gnome houses. Small things, probably more than the average person. Her lack of GH is impacting her hair. teeth. throat. ear canals. her heart size. muscle size. organ size. energy level. etc.

So the question in the followup room was, How are her other pituitary hormones functioning?

Poor Mimi...She was sent right out the door of the office and across the street to the medical center lab for another blood draw. but this time just one. And yes, she screamed from the minute he said go, all the way to the car, in the car, across the street, out of the car, walking into the lab, during the blood draw, and then, stopped when she got her sticker. (here is the save the day sticker. she attached it to my bedroom wall. and I let her keep it there. LOL)

So now we wait to hear back on the labs and a sedated MRI scheduled for January to examine the structure of the pituitary.

For all the research I've done from week 20 of my pregnancy until last week, I still could not have prepared myself. I am sad she needs shots for the next 10 years, or maybe even every day for life. I love her and I don't want her to go through all this.

I know I did absolutely all I could to find out for her what was going on. I prided myself in realizing that few kids Mimi's age get this test done (maybe 12 a year in children that young per institution...usually it's later in their childhood, say ages 8-12.) I also knew what to ask at the followup. What about her other levels? What about her eating so much? Yes, her development is right on, but she does tire out and need food, a lot. And when is the MRI?

I am proud that I joined the MAGIC Foundation a year ago, drove 1500 miles to attend the conference. Met with amazing endocrine doctors in the USA and asked their opinion.

I just have to get used to the new normal, and brace myself for a shelf fridge of medical items and a needle dispenser. I want to make the best of all the appointments that are in our future, and make those days as comfortable and happy as possible.